A small case series shows occurrence in children and supports its classification as a low-grade lymphoma.

Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma (PCSM-TCL) was provisionally included in the World Health Organization–European Organization for Research and Treatment of Cancer (WHO-EORTC) lymphoma classification of 2005. The umbrella term described disease characterized by dermal infiltrates of small- to medium-sized T cells without clinical or pathological evidence of mycosis fungoides.


This review of 10 cases identified in one tertiary academic center includes the youngest patient (age, 6 years) reported in the literature. Most of these patients (4 pediatric [age range, 6 to 16 years]; median age, 38 years; male:female ratio, 3:2) presented with a single papule, nodule, or plaque on the proximal trunk, head, or neck. No evidence of recurrence was seen in 70% of patients at a median of 2 years after treatment by excision, radiation, or some combination of excision, radiation, prednisone, and topical corticosteroids. No evidence of systemic spread was identified. Histopathology showed dermal infiltrates of small- to medium-sized lymphocytes without epidermotropism. CD4:CD8 T-cell ratios varied from 3:1 to 20:1. The numbers of eosinophils, histocytes, and mast cells varied, but a monoclonal population of T-cell lymphocytes was detected in all patients.


Citation(s):

Baum CL et al. Reappraisal of the provisional entity primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma: A series of 10 adult and pediatric patients and review of the literature. J Am Acad Dermatol 2011 Oct; 65:739.